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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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CIB3
calcium and integrin binding family member 3
Chromosome 19 · 19p13.11
NCBI Gene: 117286Ensembl: ENSG00000141977.9HGNC: HGNC:24580UniProt: Q96Q77
10PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
magnesium ion bindingcalcium ion bindingprotein bindingcalcium ion homeostasisneuroinflammatory disorderCochleosaccular degeneration - cataractcochleosaccular degeneration-cataract syndromeCataract-microcornea syndrome
✦AI Summary

CIB3 (calcium and integrin binding family member 3) is an auxiliary subunit of the mechanotransduction (MET) channel in hair cells, functioning alongside its homolog CIB2 in sensory processes 1. CIB3 contains canonical EF-hand motifs that enable calcium and magnesium ion binding, which induces conformational changes exposing hydrophobic surface areas critical for protein interactions 2. Mechanistically, CIB3 binds to transmembrane channel-like proteins (TMC1/2) through a conserved hydrophobic groove, regulating their localization and function in hair cells—a mechanism structurally similar to auxiliary subunits of voltage-gated Kv4 channels 1. Functionally, CIB3 can substitute for CIB2 in cochlear hair cells, and both proteins work jointly to regulate balance 3. Calcium modulates CIB3's interaction with TMC1 at multiple binding sites, functioning as a calcium sensor in MET channel regulation 4. Disease relevance is demonstrated by mutations in CIB genes causing hearing loss and balance disorders, with pathogenic CIB2/3 variants showing diminished calcium-binding affinities and altered TMC1 interactions 4. Additionally, CIB3 was identified through genome-wide association studies as a candidate gene associated with psychological resilience 5. Double knockout of Cib2 and Cib3 produces circling behavior in mice, highlighting the critical importance of these proteins in vestibular function 6.

Sources cited
1
CIB2 and CIB3 are auxiliary subunits of MET channels that bind to TMC1/2 through conserved hydrophobic grooves and regulate channel localization and function
PMID: 34089643
2
CIB3 and CIB2 jointly participate in regulation of balance and hearing
PMID: 40076845
3
CIB2-4 bind calcium and magnesium ions, causing conformational changes and exposing hydrophobic surface areas
PMID: 22779914
4
Calcium modulates CIB2/3 interaction with TMC1 at multiple sites and pathogenic variants show diminished calcium-binding affinities
PMID: 40000792
5
CIB3 was identified as a gene associated with resilience in genome-wide association studies
PMID: 39112778
6
Double knockout of Cib2 and Cib3 produces circling behavior demonstrating role in vestibular function
PMID: 37398316
Disease Associationsⓘ20
neuroinflammatory disorderOpen Targets
0.26Weak
Cochleosaccular degeneration - cataractOpen Targets
0.05Suggestive
cochleosaccular degeneration-cataract syndromeOpen Targets
0.05Suggestive
Cataract-microcornea syndromeOpen Targets
0.05Suggestive
Cataract with Y-shaped suture opacitiesOpen Targets
0.04Suggestive
hearing loss, autosomal dominant 87Open Targets
0.04Suggestive
hearing loss, autosomal dominant 80Open Targets
0.04Suggestive
Familial ocular anterior segment mesenchymal dysgenesisOpen Targets
0.03Suggestive
Childhood-onset autosomal recessive slowly progressive spinocerebellar ataxiaOpen Targets
0.03Suggestive
spinocerebellar ataxia, autosomal recessive 24Open Targets
0.03Suggestive
Cerebellum agenesis - hydrocephalyOpen Targets
0.03Suggestive
hydrocephaly-cerebellar agenesis syndromeOpen Targets
0.03Suggestive
early-onset non-syndromic cataractOpen Targets
0.03Suggestive
Posterior polar cataractOpen Targets
0.03Suggestive
Total congenital cataractOpen Targets
0.03Suggestive
granular corneal dystrophy type IOpen Targets
0.03Suggestive
exfoliation syndromeOpen Targets
0.03Suggestive
cataract 5 multiple typesOpen Targets
0.03Suggestive
cataract - microcornea syndromeOpen Targets
0.03Suggestive
ITM2B amyloidosisOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
TMC1Protein interaction98%TMC2Protein interaction85%KIF18AProtein interaction83%SELENONShared pathway50%TMTC2Shared pathway50%CIB4Co-mentioned in literature44%
Tissue Expression6 tissues
Bone Marrow
100%
Brain
3%
Heart
0%
Ovary
0%
Liver
0%
Lung
0%
Gene Interaction Network
Click a node to explore
CIB3TMC1TMC2KIF18ASELENONTMTC2CIB4
PROTEIN STRUCTURE
Preparing viewer…
PDB6WU7 · 1.84 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.28LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.89 [0.64–1.28]
RankingsWhere CIB3 stands among ~20K protein-coding genes
  • #17,027of 20,598
    Most Researched10
  • #13,464of 17,882
    Most Constrained (LOEUF)1.28
Genes detectedCIB3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
The Diverse Functions of the Calcium- and Integrin-Binding Protein Family.
PMID: 40076845
Int J Mol Sci · 2025
1.00
2
CIB2 and CIB3 are auxiliary subunits of the mechanotransduction channel of hair cells.
PMID: 34089643
Neuron · 2021
0.90
3
Structural insights into calcium-dependent CIB2-TMC1 interaction in hair cell mechanotransduction.
PMID: 40000792
Commun Biol · 2025
0.80
4
A meta-analysis of genome-wide studies of resilience in the German population.
PMID: 39112778
Mol Psychiatry · 2025
0.70
5
An open-source tool for automated human-level circling behavior detection.
PMID: 39245735
Sci Rep · 2024
0.60